A CASE OF RECURRENT CRANIOPHARYNGIOMA POST-OPERATIVE WITH RESIDUAL DISEASE AND GH DEFICIENCY

Abstract

INTRODUCTION
Craniopharyngioma is an uncommon intracranial tumour in childhood. Even though it is a benign tumour, recurrence of disease may occur which is commonly complicated with endocrinopathy. We present a case of recurrent craniopharyngioma post-resection with residual tumour complicated with multiple pituitary hormone deficiencies, including growth hormone (GH) deficiency.

CASE
A 7-year-8-month-old male presented with persistent headache and symptoms of increased intracranial pressure. MRI Brain showed suprasellar cystic mass. He underwent total resection of the tumour. HPE confirmed craniopharyngioma. He developed central diabetes insipidus, central hypothyroidism and ACTH deficiency post-operatively. Eleven months later, he presented with blurring of vision and increased sleepiness. Brain MRI confirmed recurrence of the tumour. Near-total-excision of the tumour was done as the tumour was adhered to the optic nerve and chiasma. After the surgery, he was under close surveillance for recurrence of disease. Annual MRI brain surveillance showed stable residual disease.

The patient is currently 12-years-old. Apart from the endocrinopathies mentioned, he is now showing signs of growth hormone deficiency such as hypothalamic obesity with weight BMI at +3.35SDS. He has poor height velocity at 3 cm/year. He has metabolic syndrome including dyslipidaemia, and fatty liver. He also has delayed bone age and poor IGF-1 level. Family counselling was done to explain the role of GH therapy for him, including the risks and benefits.

CONCLUSION
This case highlights the challenge of initiation of GH therapy, in a patient with a background history of recurrent craniopharyngioma and residual disease. Proper counselling with the patient and family is crucial to explain the clinical indications, risks and benefits of the GH therapy. A multidisciplinary approach of the management involving the paediatric endocrinologists, oncologists, neurosurgeons, radiologists, rehab physicians and dietitians together with close surveillance of primary disease are extremely important.